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Letters to the Editor
Table 3. Comparison between autoimmune myelofibrosis and myeloproliferative disorder presentation
Characteristics
Median age, IQR (years) Female (%)
Constitutional symptoms Splenomegaly
Median hemoglobin level (g/L), (IQR)
Median platelet count (109/L), (IQR)
AIMF
37 (30–49) 80
Clinical presentation
+ Absent or mild
Cytopenias at diagnosis
94 (79–106)
90.5 (75–182)
Myeloproliferative disorder8–10
66 (14-92) 39
+ +
100 (50-161)
209 (6-2466)
Median leukocyte count (109/L), (IQR)
Tear drop cells Leukoerythroblastosis Eosinophilia Basophilia
Biological autoimmune indicators (ANA, RF, hypergammaglobulinemia)
Reticulin fibrosis
Collagen fibrosis
2.65 (1.8–4)
Presence of
+/- +/- - - Yes
Bone marrow features
Predominant
Absent
9 (1-147)
+
+
+/-
+/-
Yes (early stages)
Yes (early stages)
Predominant
Osteosclerosis
Cellularity
Megakaryocyte dysplasia Intrasinusoidal hematopoiesis
Lymphoid infiltrates
Absent
Mostly hypercellular
Absent Subtle
++ (non-paratrabecular lymphoid aggregates)
+/-
Variable according to stage:
hypercellular in early pre-fibrotic stages,
normo or hypo-cellular in fibrotic stages
MK atypia and clustering ++
+/-
Plasma cells
Mutational features JAKV617F
CAL-R
Mild polytypic non-IgG4 plasmacytosis
Mutational features
Negative
-
Positive (90%) 60%
20–25%
MPL 5–10%
AIMF: autoimmune myelofibrosis; ANA: antinuclear antibodies; IQR: Interquartile range; MK: megakaryocyte; RF: rheumatoid factor.
pressive agents in 15 of 27. A second-line therapy was indicated because of incomplete response of AIMF in 7 of 29 (23%) cases and a third-line therapy in 2 of 29 (7%). For all treatment lines combined, the most frequent immunosuppressive agents associated with GC were mycophenolate mofetil (5 of 29 cases), methotrexate (4 of 29 cases) and rituximab (4 of 29 cases). Considering only cases with CR or CR lacking CBMB, the most com- mon immunosuppressive agents were mycophenolate mofetil (4 of 18), azathioprine (3 of 18) and intravenous immunoglobulins (3 of 18). Six patients were considered as non responders, and did not present any particular clinical, hematological or anatomopathological character- istics. The analysis of treatment response by treatment line is reported in Table 2. Among the 27 cases receiving GC, 18 (67%) showed CR or CR lacking CBMB. Overall, 15 patients (data available for 25 of 29 treated patients) (55,5%) cases showed GC dependency, and GC cessation was achieved in the remaining 10 (40%) with the help of immunosuppressive GC-sparing agents in only 6 of 10.
Median follow-up time was 28.5 months (range: 1 month to 18.5 years; follow-up data available for 26 of 29 cases). Six cases presented ≥1 relapse (1 due to treatment
discontinuation by the patient) (Table 2).
Treatment complications were reported for 9 of 18
cases and were mainly attributed to GC therapy. Four of the cases exhibited vascular and cutaneous fragility, three opportunistic infections (one case of pulmonary tubercu- losis, one of pneumocystis pneumonia leading to death, one without further details) and cushing-like appearance, obesity, arterial hypertension and glucocorticoid-induced diabetes in one case each.
In the absence of available response criteria for AIMF, we used an adaptation of the 2013 revised International Working Group-Myeloproliferative Neoplasms Research and Treatment (IWG-MRT) and European Leukemia Net (ELN) response criteria for myelofibrosis3 to evaluate treatment response. However, in our cases, a confirmato- ry BM biopsy was rarely performed, legitimately because of the normalization of the blood panel in response to immunosuppressive therapy and an obvious non-malig- nant context. Hence, only two cases in our study quali- fied for CR according to Tefferi et al. criteria,3 but 18 cases showed complete improvement of hematological compli- cations and symptom response without a confirmatory BM biopsy (CR lacking CBMB). The Tefferi et al. response
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